The Crucial Role of Polysomnography and Capnography in Children With Duchenne Muscular Dystrophy

Abstract

Purpose Duchenne muscular dystrophy (DMD) is a severe, progressive condition characterized by muscle degeneration and weakness, significantly affecting respiratory function. This study aimed to evaluate the presence of sleep-disordered breathing (SDB) in children with DMD and investigate the relationships between sleep and respiratory function using spirometry, sniff nasal inspiratory pressure (SNIP), and polysomnography (PSG) along with capnography.Research Question: Can low SNIP be a guide for detecting respiratory muscle involvement early and determining the right time to perform early PSG and capnography in DMD?Study Design: Prospective, observational, cross-sectional study.Methods This study included DMD patients aged < 18 years. Pulmonary function tests were conducted using spirometry and SNIP, and maximum inspiratory and expiratory pressure were measured. PSG and capnography were performed within two weeks after the pulmonary function tests, and their relationships with each other were investigated.Results The study included 44 children. Obstructive sleep apnea syndrome (OSAS) was present in 70.5% of patients, while nocturnal hypoventilation was observed in 4.5%. SNIP values were significantly lower in patients with moderate-to-severe OSAS than in those without OSAS. An SNIP value below 40 cm H2O was associated with a 92.8% prevalence of OSAS.Conclusion SNIP is a valuable, noninvasive marker for the early detection of respiratory muscle involvement and SDB in patients with DMD. This study highlights the need for early and regular respiratory monitoring in children with DMD to enhance care and quality of life.